Thursday, March 24, 2011

Malignant teratoid medulloepithelioma

Case No.1
This patient was 11 years old boy who diagnosed a protruding mass in right eye on CT findings. He received enucleation as a treatment. On gross examination, the lesion was brown colored mass with cystic change and hemorrhage, measuring 1.2 x 1.0 x 0.8 cm. The following photos are from the this mass. What do you think about the pathological diagnosis?


Final pathological diagnosis was malignant teratoid medulloepithelioma.
Information. Medulloepithelioma is a rare, malignant embryonal tumour of the central nervous system of young children, typically occurring between 6 months and 5 years of age, with about half of the cases occurring during the first 2 years. Histologically, it is characterised by papillary, tubular or trabecular arrangements of neoplastic neuroepithelium mimicking the embryonic neural tube. It may arise along nerve trunks or in the eye. Medulloepithelioma of the central nervous system is a highly malignant neoplasm with a high propensity of dissemination, while ocular medulloepithelioma usually has a benign course. Ocular medulloepithelioma most commonly affects one eye and arises from the nonpigmented epithelium of the ciliarybody. It is a slow-growing tumour, with two pathologic variants, i.e. non-teratoid and teratoid when heterologous elements, such as brain, cartilage or skeletal muscle, can be found in the tumour tissue. Both variants can be either benign or malignant. Medulloepithelioma is the second most common intraocular tumor in children, after retinoblastoma. It is usually diagnosed in the first decade of life, mostly in early childhood at a mean age of 2–4 years, although rarely it has been reported in adults. J Neurooncol (2010) 96: 443-448

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